Test Directory

Urinary Porphobilinogen is the first step in the diagnosis of acute porphyria such as acute intermittent porphyria (AIP). AIP is an autosomal dominant disorder characterized by deficiency of porphobilinogen deaminase. An acute attack usually includes gastrointestinal disturbance and neuropsychiatric disorders.

Specimen Type: Random Urine Protected from Light

Collection Container: Plastic Urine Container (Preservative Free) 

Desired Specimen Volume: 2 mL

1. Collect random urine.
2. Protect from UV light.

1. Transfer 2 mL of urine to an amber aliquot tube (screw cap).
2. If an amber aliquot tube is not available, completely wrap specimen and cap with aluminum foil.

Whenever possible, the specimen should be collected during an acute episode because porphobilinogen (PBG) may decrease rapidly upon remission. 

Transport to the laboratory frozen. 

• Not protected from light

 See patient report.

PBG may degrade when samples are exposed to UV light for extended periods, or are transported at ambient temperature.

All results should be interpreted in the context of clinical findings, relevant history, and other laboratory data.

•  Porphobilinogen, Quantitative, Random Urine